Background:Fetal alcohol spectrum disorders (FASDs) impact up to 5% of U.S. school age children; however, the burden of cardiovascular disease (CVD) in adults with FASDs remains poorly defined. We investigated associations between FASDs, cardiometabolic abnormalities, and CVD using electronic health record (EHR) data. Methods:We performed a retrospective cohort study of adults (≥18 years) receiving ambulatory care with a FASD diagnosis (n = 208, mean age 38.4±14.5, 50% female) and age- and sex-matched control patients without FASD (n = 824, mean age 41.6±14.5, 50% female). Cardiometabolic outcomes were overweight/obesity, dyslipidemia, and diabetes mellitus. Cardiovascular outcomes were congenital heart defects (CHDs), heart murmur, hypertension, conduction defects, arrhythmias, structural heart remodeling, systolic and diastolic dysfunction, heart failure, myocardial infarction (MI), stroke, and thromboembolic events. Associations were assessed using age-adjusted logistic and Poisson regression, with sex-by-diagnosis interaction testing. Multivariable logistic regression was then used to estimate the odds of cardiovascular outcomes with additional adjustment for cardiometabolic conditions. Associations between CHD and CVD outcomes were evaluated using Fisher's exact tests. Results:Adults with FASDs had a higher prevalence (p<0.05) of cardiometabolic abnormalities, including dyslipidemia, type 2 diabetes mellitus (T2DM), and the co-occurrence of multiple of these conditions (overweight/obese, HDL cholesterol < 40 mg/dL, and T2DM). CHDs were significantly more common in individuals with FASDs than controls (6% vs 1%, p < 0.001). Similarly, the FASD cohort had a higher incidence of systolic and diastolic dysfunction (6% vs. 2%), structural heart remodeling (11% vs 5%), MI (6% vs. 2%), stroke (4% vs 1%), and thromboembolic events (4% vs 1%; all p < 0.05). Significant sex-by-diagnosis interactions were observed for hypertension, arrhythmia, and heart failure, with elevated rates specific to FASD females. In individuals with FASDs, CHD diagnosis was associated with an increased incidence of conduction defects, arrhythmias, heart remodeling, heart failure, and systolic and diastolic dysfunction. Increased CVD burden in FASD adults remained significant after adjustment for BMI, composite cardiometabolic abnormalities, and hyperlipidemia. Conclusions:Adults with FASDs exhibit an increased burden of CVD not fully explained by conventional cardiometabolic risk factors. These findings support enhanced cardiovascular screening in individuals with FASDs.

Dapagliflozin has been shown to improve outcomes in adult heart failure (HF) but has not been widely evaluated in pediatrics. The present study describes dapagliflozin use and adverse events (AEs) in infants and children with HF. This descriptive, single-center retrospective review included pediatric patients with HF or recent heart transplant who were started on dapagliflozin and treated for any duration between 9/2020 and 5/2024. Descriptive statistics were used to summarize clinical characteristics, AEs, anthropometrics, and laboratory values during treatment. Of 120 patients (40.8

BACKGROUND:Risk prediction is fundamental to pulmonary hypertension (PH) guideline-based care, yet pediatric-specific risk prediction models remain limited, relying primarily on single predictors, expert opinion, or application of adult models to children. The authors developed and externally validated a data-driven 1-year risk prediction model for pediatric PH. METHODS:Pediatric patients with PH (n=345; World Symposium on Pulmonary Hypertension groups 1 and 3) enrolled in the Pediatric Pulmonary Hypertension Network Registry (2014-2020; 50.4% male; median age, 4.9 years [interquartile range, 1.9-10.3]) were split into training (80%) and test cohorts (20%). The Dutch National Registry for Pulmonary Hypertension in Childhood (n=155 [1993-2020]) and the Spanish Registry of Pediatric Pulmonary Hypertension (n=327 [2009-2023]) were used for external validation. From 176 variables, BorutaSHAP feature selection with random forest identified 16 predictors for a 1-year outcome of time to death, transplant, Potts shunt, or atrial septostomy, modeled using extreme gradient boosting. Performance was assessed with the area under the receiver operating characteristic curve, confusion matrices, calibration, and Kaplan-Meier event-free survival. RESULTS:The final model achieved an area under the receiver operating characteristic curve of 0.90 (0.79-0.97) and 99% (96%-99%) negative predictive value in testing, dividing participants into 3 groups with strong outcome discrimination. External validation showed an area under the receiver operating characteristic curve of 0.76 (Dutch National Registry for Pulmonary Hypertension in Childhood, 0.70-0.81) and 0.77 (Spanish Registry of Pediatric Pulmonary Hypertension, 0.73-0.82) with negative predictive values of 93% (93%-97%) and 96% (93%-97%), respectively. Kaplan-Meier analysis significantly differentiated outcomes by risk group. CONCLUSIONS:This multicenter, validated model provides good 1-year risk prediction in pediatric PH across World Symposium on Pulmonary Hypertension groups 1 and 3, providing a robust tool for clinical risk stratification to guide therapy and addressing a gap in pediatric PH care.

Quantification of the regurgitation fraction (RF) in pediatric patients requires cardiac magnetic resonance imaging (CMR). Three-dimensional echocardiography (3DE) can be used to measure right and left ventricular volumes and therefore has the potential to calculate the RF by comparison of stroke volumes. 3DE volume acquisitions can be acquired in only a few heartbeats, often as part of a standard pediatric study. This study compared the quantification of single valve regurgitation by 3DE with CMR, the latter serving as the gold standard. Patients < 18 years of age were included in the study if they had mild regurgitation of one valve and a biventricular circulation. Patients were excluded if they had more than mild regurgitation of another valve, or a significant shunt lesion. Full right and left ventricular volume data sets were obtained using a Philips -iE33 or EPIQ CVx machine (X5 or X7 ultrasound probe) as a part of studies done for clinical reasons. 3DE volumes and RFs were analyzed retrospectively by blinded echocardiographers using 4D Cardioview (Tomtec Imaging Systems) and compared with ventricular volumes and RF obtained by CMR. Additionally, 3DE-derived ventricular volume ratios (VVR) were analyzed for correlation and agreement with CMR. Inter- and intra-observer reliability were assessed for 3DE ventricular volumes and RF. 43 patients were included with 7 patients having two separate paired 3DE and CMR studies. The median patient age was 8.4 (IQR; 3.6–12.0) years. Anesthesia, for the CMR study, was used for 20 (40

Importance:Studies suggest that everolimus may reduce the risk of rejection, cardiac allograft vasculopathy (CAV), chronic kidney disease (CKD), and cytomegalovirus (CMV) after heart transplant. Everolimus use is controversial because of data demonstrating higher infection deaths when everolimus is introduced de novo after transplant. It is unclear whether everolimus is safe and effective when initiated at 6 months posttransplant in children, a population in which median graft survival is limited to 15 years and randomized clinical trials are lacking. Objective:To evaluate the safety and efficacy of everolimus combined with low-dose tacrolimus to prevent major adverse transplant events (MATEs) in children after heart transplant. Design, Setting, and Participants:Multicenter, randomized, open-label, clinical trial enrolling 211 patients who were alive 6 months after pediatric heart transplant at 25 US sites from February 2018 to August 2020. The last date of follow-up was April 17, 2023. Interventions:Participants were randomized to receive everolimus and low-dose tacrolimus (n = 107) or standard-dose tacrolimus and mycophenolate mofetil (n = 104) for 30 months. Main Outcomes and Measures:The primary efficacy end point was the MATE-3 score at 30 months, a validated composite ordinal end point including acute cellular rejection, CAV, and CKD. The primary safety end point was the MATE-6 score, encompassing the MATE-3 score plus antibody-mediated rejection, infection, and posttransplant lymphoproliferative disorder. Results:Among 211 children randomized, the mean age was 8.2 (SD, 6.3) years, 97 (46%) underwent transplant for congenital heart disease, and 49 (23%) were treated for rejection before 6 months. At 30 months, the mean MATE-3 score did not differ between the 2 treatment groups (mean difference, -0.32; 95% CI, -0.90 to 0.20; P = .16). The mean MATE-6 score was no higher in the everolimus group than in the mycophenolate group (baseline-adjusted mean difference, -0.40; 95% CI, -1.81 to 0.93), meeting the success criterion for safety (noninferiority margin <3). There were no differences in graft survival, MATE-free survival, or freedom from any individual MATE. Everolimus was associated with greater improvement in estimated glomerular filtration rate at 12 months (mean difference, 10.5 mL/min/1.73 m2; 95% CI, 1.09-19.91 mL/min/1.73 m2) and a lower incidence of CMV infection (hazard ratio, 0.50; 95% CI, 0.26-0.93). Conclusions and Relevance:Among 6-month pediatric heart transplant survivors, everolimus and low-dose tacrolimus did not differ from tacrolimus and mycophenolate in preventing the composite of cellular rejection, CAV, and CKD at 30 months. However, everolimus and low-dose tacrolimus appear to be safe based on the total burden of 6 MATEs and may be associated with improved kidney function and less CMV infection. Trial Registration:ClinicalTrials.gov Identifier: NCT03386539.

BACKGROUND:The effect of anticoagulation on early postoperative outcomes after pulmonary valve replacement (PVR) with bioprosthetic valves and homografts is not yet defined. We hypothesized that short-term anticoagulation would be associated with improved valve durability. METHODS:Patients undergoing PVR or right ventricle-to-pulmonary artery conduits with a bioprosthetic or homograft valve >15 mm in diameter between January 2015 and April 2021 at Boston Children's Hospital were retrospectively compared by anticoagulation status. Survival, postoperative echocardiographic data, and the incidence of pulmonary valve reintervention and prosthetic valve dysfunction were compared using multivariable Cox and logistic regression analyses adjusted for a propensity score based on anticoagulation therapy. RESULTS:Among 531 patients with a median follow-up of 1.16 years, 130 received anticoagulation therapy. Freedom from reintervention was 99%, 92%, and 86% at 1, 3, and 5 years, respectively. Adjusted for propensity score, there was no association between reintervention and anticoagulation (hazard ratio, 0.78; 95% CI, 0.18-3.47; P = .75). The propensity score-adjusted rates of valve dysfunction were 9% in anticoagulated patients and 15% in non-anticoagulated patients (odds ratio, 0.54; 95% CI, 0.18-1.56; P = .25). There was no difference in early major bleeding rates between groups (3.4% vs 2.4%, P = .59) or in other postoperative outcomes. Only 1 of 18 (6%) of all bleeding events in the anticoagulated group occurred after starting anticoagulation. CONCLUSIONS:Anticoagulation therapy appeared safe with no association with major postoperative bleeding. However, additional follow-up is necessary to assess its impact on midterm to long-term valve durability after PVR.

BACKGROUND:An electrophysiology study (EPS) with ablation is often the preferred approach to management of supraventricular tachycardia (SVT) and/or preexcitation (Wolff-Parkinson-White syndrome) in children and adolescents. There are inequities in arrhythmia management in adults, but less is known in children. We aimed to determine whether socioeconomic inequities affect referral for EPS in children with SVT or Wolff-Parkinson-White syndrome. METHODS:This retrospective observational study used the Pediatric Health Information System (49 children's hospitals' administrative data) from 2016 to 2022. We included children with SVT/Wolff-Parkinson-White syndrome at centers performing EPS and excluded those with congenital heart disease. The zip code-based Childhood Opportunity Index (COI; range 1-100) and race and ethnicity were predictors. Multivariable analysis included COI, age, race and ethnicity, census region, and preexcitation. The outcome was a procedure code for EPS. RESULTS:Among 37 674 children with preexcitation (10 165) or SVT without preexcitation (27 509), 9840 (26.1%) underwent EPS. The median COI was 58 (interquartile range [IQR], 31-81). The sample was predominantly White non-Hispanic (58%), followed by Hispanic (16%) and Black non-Hispanic (12%). Children who underwent EPS had a higher COI than those who did not (median, 59 [IQR, 34-82] versus 57 [IQR, 30-81]; P<0.001). Black non-Hispanic patients had lower EPS rates than White non-Hispanic (P<0.001) and Asian (P<0.001) patients. Hispanic patients had lower EPS rates than White non-Hispanic (P=0.001) patients. In multivariable analysis, lower COI, race and ethnicity, region, and preexcitation predicted EPS. CONCLUSIONS:In this multicenter study of pediatric patients, we found disparities in use of EPS for SVT/Wolff-Parkinson-White syndrome. In adjusted analysis, Black non-Hispanic and Hispanic children and those with a lower COI were less likely to undergo EPS.

Background The Fontan operation is associated with chronic venous hypertension, liver and renal disease, and several other sequelae. The alterative surgical approach, when feasible, a biventricular conversion (BiV), may diminish some of these long-term risks. Objectives The aim of this study was to compare long-term outcomes of patients undergoing BiV with those undergoing a destination Fontan operation. Methods We identified all patients with univentricular physiology cared for at Boston Children’s Hospital between 2007 and 2022 and divided them into those who received BiV or Fontan operations. Outcomes included 10-year incidences of modified major adverse cardiovascular events (MACE), liver dysfunction, renal dysfunction, and transplant-free survival. Outcomes in the 2 groups were compared using propensity matching. Results A total of 927 patients were evaluated, 341 BiV and 586 Fontan. Following propensity matching, 258 patients from each group were compared. There were no differences between groups in estimated 10-year freedom from MACE (P = 0.70), transplant-free survival (P = 0.70), or freedom from renal disease (P = 0.60). However, estimated 10-year freedom from liver disease was greater in BiV patients (82% BiV vs 71% Fontan, P = 0.02). Incidence rate per 100 person-years follow-up of surgical interventions and readmissions was higher among BiV patients (10.11 vs 1.85, P < 0.001 and 13.09 vs 9.6, P = 0.002), while catheter-based interventions were higher among Fontan patients (8.41 vs 4.63, P < 0.001). Conclusions Among a contemporary cohort of patients with single ventricle anatomy, BiV provide comparable long-term survival and lower risk of liver disease when compared to patients who have undergoing Fontan operations.

Long-term data on systolic and diastolic function following anomalous left coronary artery from the pulmonary artery (ALCAPA) repair are limited. We evaluated trends in these parameters and clinical outcomes over long-term follow-up. We reviewed pediatric patients who underwent ALCAPA repair from 1983–2021with ≥ 6 months of echocardiographic follow-up, assessing diastolic function indices including indexed left atrial (LA) volume, mitral inflow (E/A, E-wave deceleration time [DT]), tissue Doppler E′, and E/E′. A diastolic function score (DFS; range 0–4) was calculated from these indices, with persistent diastolic dysfunction (DD) defined as DFS ≥ 2 at ≥ 6 months post-repair. Kaplan–Meier analysis assessed time-to normalization for left ventricular (LV) ejection fraction (EF) and volumes. Regression splines analyzed trends, and Fisher’s exact test evaluated associations with a composite clinical outcome (heart failure or ventricular tachyarrhythmia). Among 37 patients, median preoperative LV EF was 26.5

Extracorporeal cardiopulmonary resuscitation (ECPR) improves survival for patients experiencing refractory cardiac arrest but is often associated with severe ischemia-reperfusion injury (IRI). Hydrogen gas (H2) has demonstrated promising preclinical efficacy in reducing IRI. The Hydrogen-FAST trial investigates the feasibility and safety of inhaled hydrogen gas as an adjunctive therapy during ECPR in patients with congenital heart disease (CHD). This phase 1 multicenter randomized controlled trial enrolls patients of any age undergoing ECPR due to refractory cardiac arrest associated with underlying cardiac morbidity. Due to the emergent nature of ECPR events, the trial leverages an Exception from Informed Consent enrollment process. Fifty-three participants will be randomized to standard care or standard care plus inhaled 2 https://clinicaltrials.gov/study/NCT05574296 .

BACKGROUND AND OBJECTIVES:Pediatric medical device (PMD) development lags behind device development for adults. We sought to identify infrastructure elements that support the conduct of PMD clinical trials (PMD-CTs). METHODS:We conducted a multistage modified Delphi process with 25 panelists who were experts on different aspects of PMD-CTs to identify and rate the importance of infrastructure elements and supporting activities. RESULTS:The panelists identified and rated the importance of 24 activities and 130 infrastructure components for conducting PMD-CTs. The panelists gave the highest importance ratings to assuring compliance with US Food and Drug Administration regulations (mean score, 5.0; IQR, 5.0-5.0; range, 5.0-5.0) and data and safety monitoring (mean score, 4.9; IQR, 5.0-5.0; range, 4.0-5.0). The infrastructure components with the highest importance ratings were "Principal investigator with clinical experience" and "Device training plan" under the activity "Implant and use device." Although some activities and infrastructure components received low importance ratings from individual panelists, all received high ratings from most panelists. CONCLUSIONS:Our panelists showed areas of agreement on what activities and infrastructure elements are important for conducting a successful PMD-CT, but panelists did not start with a common mental model of the tasks required for a PMD-CT, with some activities dismissed by a subset of panelists as unimportant or irrelevant. Lack of a shared mental model, differences in preferred terminology, and variability regarding the importance of key activities and infrastructure supports pose challenges for institutions and organizations attempting to support the development of PMDs.

INTRODUCTION:Mitral annular disjunction (MAD) is a pathologic fibrous separation of the mitral valve hinge point from the ventricular myocardium. The aims of this study were to describe the range of MAD distance by cardiovascular magnetic resonance (CMR) in children and young adults with connective tissue disorders (CTDs) versus a healthy control sample, and to assess the MAD distance as a predictor of adverse cardiovascular outcomes. METHODS:This was a retrospective, single-center study of healthy subjects and patients with Marfan syndrome, Loeys-Dietz syndrome, Ehlers-Danlos syndrome, or nonspecific CTD who underwent CMR between January 01, 2000 and January 01, 2020. The MAD distance was measured from the 2-chamber, 4-chamber, and left ventricular outflow tract views in systole and diastole and analyzed as absolute values as well as indexed to BSA and height. The primary outcome was a composite defined as the presence of significant ventricular arrhythmias, cardiac arrest, and/or death. Age-adjusted odds ratios with 95% confidence intervals and c-statistic are reported. Classification and Regression Tree analysis was performed to identify the most discriminating binary threshold to predict the occurrence of the composite outcome. RESULTS:Around 30 healthy control subjects and 254 patients with CTD met inclusion criteria. The mean ± SD age at initial CMR was 17±6years for patients with CTD and 14±3years for controls. The mean MAD distance was larger in patients with CTD compared to the control sample, and the maximum MAD distance in the control sample was 3.6 mm. Median follow-up in the CTD group was 5 years (IQR 3-11years). Thirty-four (15%) patients met the composite outcome. Systolic MAD distance was positively associated with the composite outcome. The optimal binary threshold for height-indexed maximum systolic MAD distance was 0.033 mm/cm with an event rate of 18.6% at/above threshold versus 2.6% below threshold (AUC 0.74). The association was independent of other important clinical predictors. CONCLUSION:A small MAD distance can be measured in healthy children and young adults. Children and young adults with CTD have a longer MAD distance than healthy control subjects, and a longer MAD distance is associated with adverse outcomes.

Evaluate family functioning (FF) and associations with quality of life (QOL) in a large, multicenter cohort of children and young adults with Marfan syndrome (MFS) who participated in the Pediatric Heart Network (PHN) Marfan Trial. Of the 608 patients enrolled in the PHN Marfan Trial, 359 families completed one or more of the following: the General Functioning subscale of the Family Assessment Device (FAD), the Condition Management Ability module of the Family Management Measure (FaMM), and the Pediatric Quality of Life Inventory (PedsQL) at the final trial visit at three years. The correlations between FAD and FaMM scores and patient-related factors were examined. Linear regression was used to determine the relationship between FAD, FaMM, patient-related factors, and PedsQL. 25

BACKGROUND:Optimal timing of pulmonary valve replacement (PVR) in repaired tetralogy of Fallot (rTOF) remains challenging. We hypothesized that pre-PVR artificial intelligence-enabled electrocardiogram (AI-ECG) may inform optimal PVR timing in rTOF. METHODS:rTOF PVR patients at Boston Children's Hospital (BCH) and Toronto General Hospital (TGH) with analyzable ECGs ≤3 months pre-PVR were included. Patients undergoing PVR were propensity score-matched 1:1 to non-PVR patients. Patients were partitioned into risk tertiles based on pre-PVR AI-ECG probabilities of 5-year mortality: low-, intermediate-, and high-risk. RESULTS:The PVR cohort included 605 patients (504 at Boston Children's Hospital (BCH), 101 at Toronto General Hospital (TGH); median age 20.3 [IQR, 13.6-32.0] years; median follow-up 7.5 [IQR, 4.7-10.6] years; 3.6% mortality). Pre-PVR AI-ECG risk probability was predictive of post-PVR mortality (c-index 0.77), outperforming an established imaging-based model benchmark (c-index 0.70). AI-ECG remained an independent predictor when added to the benchmark model (P < .001) with a higher c-index of 0.84. Survival was similar between low- and intermediate-risk groups (97-98% 15-year survival; P = .6), with increased mortality for the high-risk group (83% 15-year survival; P = .009). The matched cohort demonstrated that PVR was associated with increased survival overall (HR 0.28 [95% CI, 0.13-0.60], P = .001). Exploratory analyses stratified by risk group tertiles showed survival benefit associated with PVR in the intermediate-risk (HR 0.10 [95% CI, 0.01-0.86]; P = .04) and high-risk (HR 0.3 [0.1-0.7]; P = .005) groups, but not in the low-risk group (P = .8). CONCLUSIONS:AI-ECG predicts post-PVR survival in rTOF patients with a PVR survival benefit in intermediate- and high-risk, but not low-risk, groups. AI-ECG may complement imaging biomarkers to determine rTOF PVR timing.

OBJECTIVE:To determine whether the severity of pulmonary hypertension (PH), assessed at cardiac catheterization, is associated with bronchopulmonary dysplasia (BPD) severity and mortality. STUDY DESIGN:We analyzed clinical data from subjects with BPD-PH enrolled in the PPHNet Registry between 2014 and 2024. BPD severity was based on the Neonatal Research Network grading system at 36 weeks postmenstrual age. Survival and clinical associations were estimated using Cox proportional hazards regression with delayed entry-to-risk-set and competing risk methodologies. RESULTS:The study included 320 subjects (gestational age, 25.8 ± 2.0 weeks (range 22.2 to 31.6 weeks); postnatal age 5.0 months (IQR 3.3, 8.6; range birth to 12 years) at the time of PH diagnosis. The severity of BPD was 27% (grade 1), 44% (grade 2), and 29% grade 3. Twenty-two percent (n = 69) of subjects underwent cardiac catheterization within 1 month of echocardiographic diagnosis of PH. Hemodynamic features by cardiac catheterization were similar between grades of BPD severity. Five-year survival postdiagnosis was 94%, 94%, and 87%, respectively, for Grades 1, 2, and 3 BPD. Twenty-five of 320 (8%) subjects died, including 6 with pulmonary vein stenosis. CONCLUSIONS:Among subjects with BPD-PH undergoing cardiac catheterization, more deaths occurred among children with Grade 3 BPD. However, there were no substantive differences in invasive measurements of pulmonary hemodynamics based on BPD severity.

Objective Aortic valve reconstruction (AVNeo) with neocuspidization, or the Ozaki procedure with complete cusp replacement for aortic valve disease, has excellent immediate-term technical results in children and midterm results in adults. Limited midterm results of AVNeo in younger patients have been reported. We report our midterm outcomes of neocuspidization with Ozaki technique for congenital aortic and truncal valve disease. Methods A retrospective analysis was performed on all patients with congenital aortic and truncal valve disease who had a 3-leaflet AVNeo procedure at a single institution from August 2015 to June 2023. Outcome measures included mortality, surgical or catheter-based reinterventions, and echocardiography. Results In total, 74 patients aged 12.8 ± 7.3 years underwent 3 leaflet AVNeo; 31 patients had aortic regurgitation (AR), 7 had aortic stenosis (AS), and 36 patients had AS/AR. Five patients had truncus arteriosus. The aortic valve was quadricuspid in 2.7%, 46% tricuspid, 34% bicuspid, and 18% unicusp of patients. In total, 57% had previous aortic valve repairs and 9% had replacements. Preoperative echocardiography mean annular diameter was 2.03 ± 0.48 cm, and peak gradient for patients with AS/AR was 60.1 ± 24.4 mm Hg. Autologous pericardium was used in 42% (n = 31) of patients, PhotoFix bovine in 58% (n = 43) of patients, and CardioCel bovine pericardium in 2% (n = 2) of patients. In total, 71 patients had concomitant procedures, including 11 who underwent aortic root enlargement including 34 with aortic sinus enlargement. Median intensive care unit and hospital length of stay were 1.9 and 7.0 days, respectively. There were no hospital mortalities or early conversions to valve replacement. At discharge, 95% of patients had mild or less regurgitation and median peak aortic gradient was 16.9 ± 9.0 mm Hg. Over median follow-up of 3.2 years, 30 patients (40%) required aortic valve reoperation (PhotoFix, 25/43 patients, 58%; and autologous, 5/31 patients, 16%). Freedom from reoperation at 1 and 3 years for autologous was 97 and 86%, and for PhotoFix was 97% and 75% (P = .006). Freedom from a composite outcome of moderate or greater regurgitation and/or reoperation at 1 and 3 years for autologous was 89% and 67% and for PhotoFix was 92% and 54% (P = .13). Failure mode for autologous pericardium was related to calcification and for PhotoFix pericardium was dehiscence at the annular suture line. Conclusions The AVNeo procedure has acceptable short-term results in all patients but especially poor midterm results with PhotoFix bovine pericardium. AVNeo could be considered with caution for valve reconstruction in congenital patients if native autologous pericardium is available.

Background Reduced biventricular global function index (BVGFI) is associated with adverse outcomes in repaired tetralogy of Fallot (rTOF). The change in BVGFI associated with pulmonary valve replacement (PVR) is unknown. Objectives To characterize BVGFI following PVR in rTOF and identify pre-PVR factors associated with severely depressed post-PVR BVGFI. Methods Single-center retrospective cohort study of rTOF patients with a cardiac magnetic resonance (CMR) examination within 1 year before and 2 years after their first PVR and no interval cardiac procedures (n=133). CMR parameters between rTOF and normal controls (n=136) were compared. BVGFI was categorized as normal (≥46.2), mild-moderately depressed (40.0-46.1), or severely depressed (<40.0). Pre- vs. post-PVR changes and pre-PVR correlates of severely depressed post-PVR BVGFI were explored. Results When adjusted for age and sex, pre-PVR BVGFI was lower in patients with rTOF compared to controls (47.7±0.6 vs. 56.0±0.5, p<0.001), with 48% of rTOF patients having subnormal pre-PVR BVGFI. Overall, compared with pre-PVR values, mean BVGFI did not change after PVR (46.6±7.7 vs. 45.6±6.7, p=0.28), while RVGFI declined from 49.6±10.2 pre-PVR to 46.1±9.0 post-PVR (p=0.003). Among patients with normal pre-PVR BVGFI (n=69), 64% remained normal, whereas 36% declined. Of those with severely depressed pre-PVR BVGFI (n=24), 50% remained severely depressed, and only 4% achieved normalization of BVGFI after PVR. Factors independently associated with severely depressed post-PVR BVGFI were lower pre-PVR BVGFI, male sex, moderate or severe pulmonary regurgitation (PR), and higher left ventricular end-systolic volume index (LVESVi). Type of pre-PVR hemodynamic load was not associated with the odds of severely depressed BVGFI post-PVR. Conclusions BVGFI is depressed in about half of rTOF patients pre-PVR and did not significantly change post-PVR remaining stable in most patients. Lower pre-PVR BVGFI, male sex, moderate or severe PR, and higher LVESVi are independently associated with severely depressed post-PVR BVGFI.

Background: Patients with hypoplastic left ventricles (LV) who undergo volume-loading procedures (recruitment, biventricular [BIV] repair) are at risk for adverse outcomes, including heart failure and death. We investigated pre-BIV LV shape as a predictor of outcome after BIV repair in patients with hypoplastic LVs. Methods: Baseline and post-recruitment cardiac magnetic resonance imaging and computed tomography data were analyzed in patients with hypoplastic LV (<50 mL/m(2)). Statistical shape modeling (SSM) was utilized to generate a model of the shape and variability of LVs. Traditional measures of LV sphericity and eccentricity were also measured. Major adverse cardiovascular events (MACE) included heart failure, transplant, and death. Results: Of 95 patients with baseline mean LV volume 29 +/- 13 mL/m(2), 45/95 (47%) had a right dominant atrioventricular canal defect, 31/95 (33%) had a variant of hypoplastic left heart syndrome, and 18/95 (19%) had endocardial fibroelastosis (EFE). A wide variation in LV shape was found by SSM, and shape modes were associated with right ventricle (RV) and LV size, and diagnosis. BIV repair was achieved in 74/95 (78%) patients; 13/74 (18%) of BIV patients had MACE. Predictors of MACE following BIV repair included EFE, higher RV mass index, and higher RV end-diastolic volume index. No baseline or post-recruitment LV shape parameter was associated with the outcome after BIV repair. Conclusion: The shape model of hypoplastic LVs demonstrated a wide array of LV shapes. LVs gained sphericity and size and lost eccentricity with recruitment. Though the ventricles changed shape with recruitment, no specific LV shape characteristic at the baseline or post-recruitment stage was predictive of decision to proceed with BIV repair or outcome. Higher RV mass and volume may represent new biomarkers that predict outcomes following BIV repair in patients with hypoplastic LV. Further investigation could determine the reproducibility of these findings.